Ratchets, red cells, and metastability
Tóm tắt
Sickle cell disease is a genetic disorder in which a negatively charged glutamic acid is replaced by a hydrophobic valine on the surface of the hemoglobin molecule, leading to polymerization of the deoxygenated form, and resulting in microvascular obstruction. Because of the high volume occupancy under which polymerization occurs physiologically, this process has been an exemplar in the study of excluded volume effects on assembly. More recently, we have identified yet another type of crowding effect involving the obstruction of the ends at which the polymers grow as a consequence of the dense arrays in which these polymers form. This makes such solutions metastable, and leads to Brownian ratchet behavior in which pressure is exerted outward when the gel occupies a finite volume, as in an emulsion or red cell. Such behavior is capable of holding sickled cells in place in the microcirculation against weak pressure differentials (hundreds of Pa), but not against the typical pressures found in vivo.
Tài liệu tham khảo
Aprelev A, Weng W, Zakharov M, Rotter M, Yosmanovich D, Kwong S, Briehl RW, Ferrone FA (2007) Metastable polymerization of sickle hemoglobin in droplets. J Mol Biol 369(5):1170–1174
Aprelev A, Liu Z, Ferrone FA (2011) The growth of sickle hemoglobin polymers. Biophys J 101(4):885–891. doi:10.1016/j.bpj.2011.05.064
Aprelev A, Stephenson W, Noh HM, Meier M, Ferrone FA (2012) The physical foundation of vasoocclusion in sickle cell disease. Biophys J 103(8):L38–L40. doi:10.1016/j.bpj.2012.09.003
Bishop MF, Ferrone FA (1984) Kinetics of nucleation controlled polymerization: a perturbation treatment for use with a secondary pathway. Biophys J 46:631–644
Cabrales P, Tsai AG, Intaglietta M (2004) Microvascular pressure and functional capillary density in extreme hemodilution with low- and high-viscosity dextran and a low-viscosity Hb-based O2 carrier. Am J Physiol Heart Circ Physiol 287(1):H363–H373. doi:10.1152/ajpheart.01039.2003
Daniels DR, Wang JC, Briehl RW, Turner MS (2006) Deforming biological membranes: how the cytoskeleton affects a polymerizing fiber. J Chem Phys 124(2):024903
Dunn AC, Zaveri TD, Keselowsky BG, Sawyer WG (2007) Macroscopic friction coefficient measurements on living endothelial cells. Tribol Lett 27:233–238
Eaton WA, Hofrichter J (1987) Hemoglobin S gelation and sickle cell disease. Blood 70:1245–1266
Eaton WA, Hofrichter J (1990) Sickle cell hemoglobin polymerization. Adv Protein Chem 40:63–280
Ferrone FA, Rotter MA (2004) Crowding and the polymerization of sickle hemoglobin. J Mol Recognit 17(5):497–504
Ferrone FA, Hofrichter J, Eaton WA (1985a) Kinetics of sickle hemoglobin polymerization II: a double nucleation mechanism. J Mol Biol 183:611–631
Ferrone FA, Hofrichter J, Eaton WA (1985b) Kinetics of sickle hemoglobin polymerization I: studies using temperature-jump and laser photolysis techniques. J Mol Biol 183:591–610
Galkin O, Nagel RL, Vekilov PG (2007) The kinetics of nucleation and growth of sickle cell hemoglobin fibers. J Mol Biol 365(2):425–439
Kaul DK, Fabry ME, Nagel RL (1989) Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiological implications. Proc Natl Acad Sci USA 86(9):3356–3360
Liao D, Jiang J, Zhao M, Ferrone FA (1993) Modulated excitation of singly ligated carboxyhemoglobin. Biophys J 65:2059–2067
Liao D, Martin de Llano JJ, Himanen J-P, Manning JM, Ferrone FA (1996) Solubility of sickle hemoglobin measured by a kinetic micromethod. Biophys J 70:2442–2447
Ogston AG (1970) On the interaction of solute molecules with porous networks. J Phys Chem 74:668–669
Peskin CS, Odell GM, Oster GF (1993) Cellular motions and thermal fluctuations: the Brownian ratchet. Biophys J 65(1):316–324. doi:10.1016/S0006-3495(93)81035-X
Pries AR, Secomb TW, Gessner T, Sperandio MB, Gross JF, Gaehtgens P (1994) Resistance to blood flow in microvessels in vivo. Circ Res 75(5):904–915
Ross PD, Hofrichter J, Eaton WA (1977) Thermodynamics of gelation of sickle cell deoxyhemoglobin. J Mol Biol 115:111–134
Shapiro DB, Esquerra RM, Goldbeck RA, Ballas SK, Mohandas N, Kliger D (1995) Carbon monoxide religation kinetics to hemoglobin S polymers following ligand photolysis. J Biol Chem 270(44):26078–26085
Sunshine HR, Hofrichter J, Eaton WA (1979) Gelation of sickle cell hemoglobin in mixtures with normal adult and fetal hemoglobins. J Mol Biol 133:435–467
Weng W, Aprelev A, Briehl RW, Ferrone FA (2008) Universal metastability of sickle hemoglobin polymerization. J Mol Biol 377(4):1228–1235. doi:10.1016/j.jmb.2008.01.083
Zakharov MN, Aprelev A, Turner MS, Ferrone FA (2010) The microrheology of sickle hemoglobin gels. Biophys J 99(4):1149–1156. doi:10.1016/j.bpj.2010.04.079